Glutathione in fibroblasts from normal and cystinotic children |
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Authors: | Beatrice States Karen Scardigli Stanton Segal |
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Affiliation: | 1. Division of Biochemical Development and Molecular Diseases, Children''s Hospital of Philadelphia, Medical School of the University of Pennsylvania, Philadelphia, Pennsylvania, USA;2. Department of Pediatrics and Medicine, Medical School of the University of Pennsylvania, Philadelphia, Pennsylvania, USA |
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Abstract: | Intracellular levels of GSH and GSSG were compared in fibroblasts initiated from skin biopsies from normals and cystinotics. The concentration of GSH was essentially the same in both cell lines. However, the intracellular GSSG in cystinotic cells averaged less than half of that in normal cells. The ratio of intracellular GSH:GSSG was 10.6 in cells from cystinotics as compared with 4.7 in cells from normals. A significant distortion in the ratio of GSH:GSSG serves as another marker of the derangement of sulfur-amino acid metabolism in cystinosis. Whether this effect is primary or secondary remains to be elucidated. |
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